OBJECTIVE: 1) Define the role of preoperative investigationsfor an accurate diagnosis of site/extension of the skull basedefect (SBD). 2) Identify different solutions in performingduraplasty, choosing among available techniques/materials. 3)Evaluate the efficacy and safety of the endoscopic endonasalapproach in pediatric age.

METHOD: From 2000 to 2010, 26 patients (14 males, 12females, mean age 8.9 years) affected by SBD referred to ourInstitute. The diagnostic work-up provided endoscopic exam-ination and neuroimaging with CT and MR. Intraoperativeintrathecal fluorescein test was performed during surgery todisplay the defects site/s and ensure a watertight duraplasty.Follow-up was carried out with periodic endoscopic and MRevaluation.

RESULTS: Pediatric SBDs represent 9.6% of our experiencein cerebrospinal fluid leaks treatment. 14 patients were affectedby spontaneous defects, 12 by traumatic defects (2 iatrogenic,10 accidental). 21 patients were treated with a pure endoscopictechnique, 5 patients underwent a combined, endoscopic en-donasal and craniotomic surgical approach. Duraplasty wasperformed with single/double layer (11) or multilayer (15)technique, depending on the site. The most employed materialswere autologous. No intra- and postoperative complicationswere observed. Mean follow up was 5.5 years.

CONCLUSION: SBDs are rare conditions, which requiresurgical treatment in order to avoid serious complications. Ourexperience highlights that an accurate preoperative assessmentof the defect is mandatory to guarantee the proper selection ofthe surgical technique. The use of dedicated instruments grantsa minimally invasive surgical approach, which decreases therisk of functional/aesthetic strings. Larger and longer studiesare needed.

The Effectiveness of Childhood Tonsillectomy

Janet Wilson, MD, FRCSEd, FRCSEng (presenter);Sean Carrie, FRCS; Catherine Lock, MA, PhD; MartinEccles, MD, FMedSci, FRCP; Nick Steen, PhD

OBJECTIVE: To assess the clinical and cost effectiveness oftonsillectomy/adeno-tonsillectomy in children aged 4-15 yearswith recurrent sore throats.

METHOD: Design: A pragmatic randomized controlled Trial(Trial) and a parallel, non-randomized, patient preference arm(Cohort). Participants: 1546 children aged 4 to 15 years re-ferred to secondary care with recurrent sore throats assessed;1015 eligible; 268 enrolled to the Trial; 461 to the Cohort.Setting: 5 secondary care UK otolaryngology departments.The Trial surgical treatment arm - (adeno) tonsillectomy within12 weeks of randomization. Control � conventional medicaltherapy. The primary outcome was the reported number ofepisodes of sore throat in the two years after study enrollment.

An intention to treat analysis was performed according to theoriginal protocol, followed by an as-treated analysis, incorpo-rating Trial plus Cohort patients.

RESULTS: Children randomized to surgery had fewer sorethroats [incidence rate ratios year 1: 0.70 (95% CI: 0.61, 0.80),year 2: 0.54 (95% CI: 0.42, 0.70)]. Estimated effect of tonsil-lectomy by intention to treat � 3.5 fewer sore throats in 2 y.There was a 26% cross over from the medical to the surgicalarm. The as treated analysis showed surgery prevented anaverage of over 8 sore throats in 2 y if performed within 10weeks of consultation.

CONCLUSION: Tonsillectomy is effective at reducing therates of reported sore throats. The maximum benefit of inter-vention is achievable by operating as soon as possible afterconsultation. If there is a high social or educational impact oftonsillitis, this is an important consideration.

The Incidence of Hearing Loss in Trisomy 21

Albert Park, MD (presenter); Paul Stevens, BS;Nancy Hohler, MA; Richard Harward, AuD; NanetteSturgill, MS

OBJECTIVE: 1) Determine the incidence and type of hearingloss in trisomy 21 patients. 2) Ascertain the time for diagnosisof hearing loss in trisomy 21 patients who fail their newbornhearing screening tests.

METHOD: Three hundred fourteen Down syndrome patientsborn in Utah between January 2002 to December 2006 wereidentified using the Utah Department of Health newborn hear-ing screening and Down syndrome registry. Newborn hearingtests, follow up studies and exams leading to a diagnosis ofnormal or impaired hearing were reviewed retrospectively.

RESULTS: Seventy-two of trisomy 21 children (22.9%) hadhearing loss. Thirteen (4.1%) were diagnosed with sensorineu-ral hearing loss (SNHL); fifty-nine (18.8%) were diagnosedwith conductive hearing loss (CHL). Eleven of thirteen pa-tients with SNHL failed their newborn hearing (NBH) screen(2 unavailable). One had a unilateral profound SNHL. Twelvechildren had bilateral SNHL (mild to profound). Seven werediagnosed within the first 3 months of life and five had hearingaids within a month. The average time to diagnosis was 263days (SD 388 days).Thirty-eight patients with CHL passedtheir NBH screen and eighteen failed (3 not available). Twen-ty-four patients improved their hearing with tubes. The averagetime to diagnosis for those who failed their initial NBH screenwas 330 days (SD 355).

CONCLUSION: Trisomy 21 children have a relatively highincidence of both SNHL and CHL. Time to diagnosis for bothSNHL and CHL took longer than the recommended guidelinesrecommended in 2007 from the Joint Committee on InfantHearing.

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